Congenital Heart Disease

1
Congenital Heart Disease

• Dr Rajesh Kumar
• MD (PGI), DM (Neonatology) PGI, Chandigarh, India
• Rani Children Hospital, Ranchi

2

• Where there is life there is hope

3
Incidence

• Incidence 7.5 per 1000 live births
• Based on ECHO at least 3-4 times higher
  incidence
• 2.7 per 1000 live birth requires heart surgery
• Gordon Avery 5th edition

4
Causes of cardiac failure

• Cardiac
• Structural
• Arrythmia
• Myocardial dysfunction
• Extracardiac compression

• Non-cardiac
• Preload (ARF)
• Afterload (HT)
• O2 carrying capacity (anemia)
• Demand (sepsis)

5

• Presenting on day 1-2
• Presenting after going home
• Left to right shunts

6
Top 5 diagnoses during neonatal period

• 1st week
• TGA, HLH, TOF, CoA, VSD
• 2nd week
• CoA, VSD, HVH, TGA, TOF
• 3-4th week
• VSD, CoA, TOF, TGA, PDA

7
Signs and symptoms of CCF

• Tachycardia

• Venous congestion
• Right side
• Hepatomegaly
• Ascitis
• Pleural effusion
• Edema
• Left side
• Tachypnea
• Retactions
• Crepitations
• Pul. edema

• Low cardiac output
• Acute
• Pallor
• Sweating
• Cool extremities
• capillary refill
• Altered sensorium
• Chronic
• Feeding difficulty
• Fatigue
• Poor growth

8
Neonatal cardiac physiology

• The transformation from fetal to neonatal
  circulation involves two major changes
• A marked increase in systemic resistance.
• caused by loss of the low-resistance placenta.
• 2. A marked decrease in pulmonary resistance.
• caused by pulmonary artery dilation with the
  neonates first breaths.

9
Fetal cardiac physiology

• Fetal circulation
• Blood flows from the placenta
• ? IVC
• ? RA
• ? through the PFO
• ? LA
• ? LV

? ascending aorta ? brain
? returns via the SVC
10
Fetal cardiac physiology

• Fetal circulation
• From the SVC
• ? RA
• ? RV

? pulm aa ? through the PDA
? descending aorta ? lower
extremities and placenta
11
Fetal cardiac physiology

• Fetal circulation
• Only a very small amount of blood is directed
  through the right and left pulmonary aas to
  the lungs.

12
Neonatal cardiac physiology

• Neonate circulation
• The transformation to neonatal circulation occurs
  with the first few breaths.
• The two remaining remnants of the fetal
  circulation are a patent foramen ovale...

and ductus arteriosus.
13

• During neonatal period systemic flow is combined
  LV and RV output
• After birth RV blood goes to lung and LV blood
  goes to body
• Example Valvular atresias

14
Congenital Heart Disease

• Neonates with CHD often rely on a patent ductus
  arteriosus and/or foramen ovale to sustain life.
• Unfortunately for these neonates, both of these
  passages begins to close following birth.
• The ductus normally closes by 72hrs.
• The foramen ovale normally closes by 3 months.

15
CHD

• In the presence of hypoxia or acidosis (generally
  present in ductus-dependent lesions), the ductus
  may remain open for a longer period of time.
• As a result, these patients often present to the
  ED during the first 1-3 weeks of life.
• i.e. as the ductus begins to close.

16
Cardiac disease presenting within 24 hours

• CCF
• Structural
• LVOT obstruction
• Myocardial dysfunction
• HIE TR
• Acidosis (acute LVF)
• Arrythmia
• SVT, CHB
• Extracardiac compression

• Cyanosis
• TGA
• TOF
• ECD
• Murmur
• VSD
• TGA with VSD
• TOF

17
LVOT obstruction

• Case 1 Term neonate had
• tachycardia and tachypnea since birth,
  Hepatomegaly
• CXR Congested lung fields
• ECHO Critical AS, died on day 4
• Case 2 Term baby,
• respiratory distress since birth, hepatomegaly,
• CXR congested Lung field,
• ECHO hypoplastic left heart, died on day 1

18
Acute LVF

• Born to mother with MSL
• Asphyxia, had gastric bleeding at 3 hours of age
• Seizure at 3 ½ hours of age, shifted to RCH
• Suddenly had frothing, SpO2 down, intubated, put
  on ventilator

19

20

• Had acute LVF, Pulmonary edema
• Had severe acidosis, corrected with sodabicarb
• Was on ventilator for 5 days
• Was on oxygen for 12 days
• In follow up normal development

21
Congenital heart block

• Baby diagnosed as congenital heart block
• Developed CCF on Day 2
• Temporary pacing was done
• Later Permanent pacemaker was implanted

22
TGA

• Born at Apollo by LSCS, Had tachypnea since birth
• SpO2 87-92 on maximum oxygen
• ECHO suggestive of TGA
• Referred to Escorts, on same day
• Septostomy was done, later Arterial switch was
  done

23
Cardiac disease presenting on 2-14 day

• CCF
• Structural
• LVOT obstruction
• Myocardial dysfunction
• ALCAPA
• Cardiomyopathy
• Acidosis (acute LVF)
• Arrythmia
• SVT, CHB
• Extracardiac compression

• Cyanosis with CCF
• TGA
• ECD
• Cyanosis
• TOF
• PA, TA
• Murmur
• VSD
• TGA with VSD
• TOF

24
Top 5 diagnoses during neonatal period

• 1st week
• TGA, HLH, TOF, CoA, VSD
• 2nd week
• CoA, VSD, HVH, TGA, TOF
• 3-4th week
• VSD, CoA, TOF, TGA, PDA

25

• 15 days old baby
• Came with respiratory distress and cyanosis
• Had CCF
• ECHO Transposition of great arteries with VSD
• CCF managed and referred for Arterial switch

26
TGA with VSD operated
27
TGA

• 2 Kg baby was admitted on day 12 with
  phenobarbitone overdose
• Found to have mild cyanosis
• ECHO TGA with VSD
• Operated had complicated post op period
• Remained in NICU for 1 month

28
ALCAPA

• 10 Days old baby with tachypnea, CXR showed
  congestion, ECHO MR TR
• Improved on decongestives, diagnosed as
  cardiomyopathy, IEM workup normal
• Repeat ECO ALCAPA
• Operated at Escorts, now asymptomatic

29
Classifying CHD

• There are many different classification systems
  for CHD.
• None are particularly good.
• Pink/Blue/Grey-Baby system
• Pink Baby Left to right shunt
• Blue Baby Right to left shunt
• Grey Baby LV outflow tract obstruction

30
Cyanosis

• On Day 1-2
• TGA
• PA
• TA
• TOF with severe RVOT obstruction
• CXR and ECG helpful
• Ductus dependent

• 2-10 days
• TGA
• TOF
• TA
• Truncus

31
Diagnosis of CCF ECG

• More useful in D/D of cyanotic newborn with pul
  blood flow

-90
Tricuspid atresia
0
180
Pul atresia with intact vent septum
TOF, Pul stenosis
90
32
Hyperoxia test

• 100 oxygen for 10 min
• PaO2 should not increase by gt30 in CCHD
• In PA, TA PaO2 will be lt 60 mmHG
• In AdmixtureTGA, Truncus PaO2 will be lt250 mm
  Hg
• By pulse oxymetry if spo2 increases by gt 10 no
  CCHD

33
CYANOSIS
Give 100 O2
RA PaO2 gt220Pulmonary Disease
RA PaO2 lt220
History, Exam, ECG, ECHO
Probable Pulmonary disease
Probable Cyanotic Heart
Optimise ventilation
RA PaO2 gt220
RA PaO2 lt220
PPHN / Lung disease / Cyanotic Heart
SaO2 gt 70, perfusion normal
SaO2 lt 70, perfusion decreased
PPHN/ Lung disease
No PDA dependent
? PDA dependent
Consider PGE1
34
1 year, 9 kg
35
Tetralogy of Fallot

• Characterized by
• Pulmonary art OTO
• RV hypertrophy
• VSD
• Over-riding aorta
• Anteriorly placed conal septum
• With severe pulmonary OTO... Blood flow to the
  lungs may be highly ductus-dependent.

36
Tetralogy of Fallot

• The classic CXR finding in TOF is the boot-shaped
  heart.

• Pulmonary vasculature is typically decreased.

37
Tetralogy of fallot

• VSD, Pulmonary outflow obstruction, RVH,
  Overriding of aorta
• Anteriorly placed conal septum

38
Clinical Presentation

• Wide spectrum primarily related to the degree of
  RVOT obstruction, the anatomy of the pulmonary
  arteries, and presence/absence of other sources
  of pulmonary blood flow
• Typically, patients with TOF present with a
  murmur and variable degrees of cyanosis,
  depending on the degree of pulmonary stenosis.
• The murmur is due to turbulence of flow caused by
  the pulmonary stenosis., not due to VSD.
• Other findings might include clubbing of the
  digits (not at lt 3 mos), increased RV impulse, a
  single S2, and sometimes an ejection click.

39
TOF management

• May deteriorate after PDA has closed is severe
  RVOT obstruction (will require PGE1)
• Single tet spell is indication of surgery
• Pulmonary valve and pulmonary artery anatomy is
  important
• Primary corrective surgery is done
• Without surgery 1 year mortality is 35

40
Transposition of the Great Arteries

• TGA is the most common cyanotic lesion presenting
  in the first week of life.
• Anatomically
• RV ? aorta
• LV ? pulmonary aa

• To be compatible with life, mixing of the two
  circulations must occur via an ASD, VSD, or PDA.

41
Transposition of the Great Arteries

• The CXR findings in TGA are typically less
  dramatic than in TOF.
• Pulmonary vasculature is typically increased.

42
TGA Treatment

• Prostaglandin El to maintain patency of the DA
• Balloon atrial septostomy if FO is restrictive
• Arterial switch operation with reimplantation of
  coronary arteries

43
Blue Baby (R ? L shunt)

• Hypoxia and cyanosis (unresponsive to oxygen) in
  the neonatal period suggests a ductus-dependent
  lesion.
• Treatment is a prostaglandin-E1 (PGE1) infusion.
• Dosing discussed momentarily
• This should obviously be accompanied by urgent
  Peds Cardiology and PICU consultation.

44
Grey Baby (LVOTO)

• Left-ventricular outflow tract obstructions
  (LVOTOs) lead to cyanosis, acidosis, and shock
  early in the neonatal period.
• Complete obstruction is universally fatal unless
  shunting occurs through an ASD, VSD, or PDA.
• Examples of these lesions include
• Severe coarctation of the aorta
• Hypoplastic left heart syndrome (HLHS)

45
Grey Baby (LVOTO)

• Treatment
• Any neonate presenting with shock unresponsive to
  fluids /- pressors has a LVOTO until proven
  otherwise.
• As with the Blue babies, appropriate management
  is an urgent PGE1 infusion and emergent
  consultation.

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CCF on Day 1-2

• Medically treatable
• SVT
• Severe TR
• Myocardial failure
• ALCAPA

• Practically non-tretable, requires PGE1

48
CCF 3-10 days

• Duct dependent LVOT obstruction
• CoA
• HLHS
• Critical AS

49
Ductus dependent CHD

• Severe right ventricular obstruction
• TOF with severe pulmonary stenosis / atresia
• Critical PS
• PA with intact septum
• TA

• Severe left ventricular obstruction
• Preductal coarct
• Interrupted aortic arch
• Critical AS
• HLH
• Mitral atresia

50
Pneumopericardium
51
1 year, 9 kg
52
TGA with VSD operated
53
TGA

• 2 Kg baby was admitted on day 12 with
  phenobarbitone overdose
• Found to have mild cyanosis
• ECHO TGA with VSD
• Operated had complicated post op period
• Remained in NICU for 1 month

54
Prostaglandin-E1

• PGE1 promotes ductus arteriosus patency.
• Use an IV infusion at 0.05-0.1 ug/kg/min.
• A response should be seen within 15 min.
• If ineffective, try doubling the dose.
• If effective, try halving the dose.
• The lowest possible dose should be used as
  adverse-effects of PGE1 can include
• - fever - flushing
• - diarrhea - periodic apnea (be ready to
  intubate)

55
Myocardial dysfunction
56

• There is abrupt increase in the LV work after
  birth
• becomes sole supplier to systemic circulation,
• volume increases
• 25 infants with myocardial disease presents in
  the 1st week of life

57

• LCAPA should be considered in all children with
  dilated cardiomyoparhy
• ECG pattern of anterolateral MI

58
Arrythmia
59
Sinus arrhythmias

• Sustained HR lt70 is abnormal
• Causes of bradycardia
• Non Cardiac
• Hyperkalemia
• HIE
• Hypothyroidism
• GER
• Cardiac
• Heart block
• Long QT syndrome

60
Case study

• Term newborn, Wt 3.0 Kg
• Antenataly suspected congenital heart block
• At birth heart rate 50 per minute, Echo normal,
  ECG s/o CHB
• Developed tachypnea and retraction on day 3
• Required temporary pacing followed by permament
  pace maker implant
• Well till 1 year of life

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Causes of CCF Cardiac-arrythmia

• Congenital heart block
• Supraventricular tachycardia
• Ventricular tachycardia

63
Complete Heart Block

• AHA recommendation for elective cardiac pacing
• With normal heart lt50 per min
• With Structurally abnormal Heart lt70 per min

64
Supraventricular tachycardia

• Digoxin
• Propanalol
• Adenosin
• Amiodarone

65
Ventricular tachycardia

• Lignocaine drip

66
Prostaglandin E1

• Useful in ductal dependant CHD
• Best before 96 hours after birth
• Dose 0.5 0.2 mg/kg/minute
• Presentation ALPOSTIN, 1 ml ampoule, 1ml500mg
• C/I PFC, infradiafragmatic TAPVC
• Side effects Apnea

67
Correction of metabolic derangements

• Correct metabolic acidosis
• 2 ml/kg bolus, later by ABG report
• Correct hypoglycemia
• 2 ml/kg of 10 dextrose
• Correct hypocalcemia
• 2 ml/kg calicium gluconate over 5 minutes

68
Improved oxygen delivery

• Oxygen content of blood
• Hb X saturation X 13.6 0.0031 X PaO2
• Start oxygen
• Blood transfusion if HB lt10-13 gm
• Iron supplementation

69
Aim

• What are the causes of CCF in neonate?
• How to diagnose CCF in a neonate?
• What are the different investigations required?
• What is the treatment?

70
Definition

• Heart is unable to meet the metabolic demands of
  the tissues

71
Diagnosis of CCF

• Clinical
• Radiographic findings
• Laboratory findings

72
Diagnosis of CCF X-ray

• To rule out primary pulmonary disease
• Magnitude of pulmonary blood flow
• Cardiac size
• Cardiac shape (boot shaped, egg on side, snow
  man)

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Diagnosis of CCF Echo

• Rules out associated significant heart disease in
  pt with pulmonary disease
• Doppler echo is preffered
• Operator dependant
• Examination of extracardiac structure is limited

75
Diagnosis of CCF Cardiac catheterisation

• Necessary to delineate vascular anatomy before
  surgery in some cases

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What is the definition and the major features of
Transposition of the Great Arteries?
79

• Definition the aorta arises from the morphologic
  right ventricle and the pulmonary arises from the
  morphologic left ventricle. This results in two
  circulations in parallel rather than the normal
  series circulations with resultant severe
  hypoxemia after birth.
• Without some mixing at either the atrial,
  ventricular, or ductal levels, these infants die
  early in infancy. Prior to current intervention
  methods, mortality was 50 by one month of age
  and 90 by 6 months.
• TGA occurs most commonly with intact ventricular
  septum, and infants usually present with cyanosis
  unresponsive to 02 after the DA closes.

80

• 21 male predominance (Big Blue Baby Boys)
• Increased RV impulse (sometimes)
• Single S2
• Frequently no murmur

81
What is the definition and what are the Major
features of Tricuspid Atresia?
82

• Definition complete atresia of the tricuspid
  valve and absence of direct communication between
  RA and RV
• Because of this, there is an obligatory ASD
• Numerous types and subtypes based on relationship
  of great arteries, presence/absence/size of VSD,
  and presence/absence of PS or pulmonary atresia
• This discussion limited to tricuspid atresia with
  intact ventricular septum or small VSD
• Results in cyanosis of infant after FDA closes
• Prominent LV impulse (RV is hypoplastic)

83

• May be no significant murmur after DA closes
• VSD murmur (if defect present)
• Normal S1
• Single S2

84

• ECG frequently diagnostic with the following
  abnormalities
• CXR enlarged heart with prominent RA and
  decreased pulmonary vascular markings
• Echocardiography confirms diagnosis and
  associated lesions
• Cardiac catheterization rarely necessary in
  neonate unless ASD is restrictive.
• Performed later in preparation for surgery after
  the neonatal period.

85

• Prostaglandin El to reopen ductus or maintain
  ductal patency
• Subclavian artery to pulmonary artery
  (Blalock-Taussig) shunt to assure adequate
  pulmonary blood flow as neonate and young infant
• Bidirectional superior vena cava to right
  pulmonary artery (Glenn) shunt at 6-12 months
  of age
• Modified Fontan procedure later in childhood

86
What is the Definition and Major features of a
Total Anomalous Pulmonary Venous Connection
(TAPVC)?
87

• Definition all pulmonary veins connect
  anomalously to the systemic venous circulation.
  Therefore, there is complete mixing of pulmonary
  and systemic venous blood at the level of the RA.
• May be associated with either increased Qp (with
  minimal or no clinical cyanosis) or decreased
  Qp (usually with severe cyanosis), depending on
  absence or presence of obstruction to pulmonary
  venous return
• TAPVC always associated with an interatrial
  communication

88

• Primitive foregut gives rise to lungs, larynx,
  and tracheobronchial tree
• Lung buds share common vascular plexus, the
  splanchnic plexus, with other foregut derivatives
• Early on, lung buds drain through right and left
  common cardinal and umbilicovitelline systems of
  veins
• Splanchnic plexus differentiates into primitive
  pulmonary vascular bed and drains pulmonary
  venous blood. However, it remains in
  communication with cardinal and umbilicovitelline
  veins until later in development
• Right common cardinal system ultimately gives
  rise to right SVC and azygous vein
• Left common cardinal system ultimately gives rise
  to left SVC and coronary sinus
• Umbilicovitelline system becomes the IVC, DV, and
  portal vein
• Initially no direct communication with the
  developing heart

89

• Supracardiac (55)
• Most common
• CPV connects to anomalous vertical vein to LIV
  which drains into RSVC
• Obstruction uncommon
• TAPVC to RSVC uncommon and usually associated
  with complex cardiac malformations
• Cardiac (30)
• To CS or posterior RA
• Obstruction occurs in 20 of cases
• Infracardiac (13)
• Almost always obstructed
• Pulmonary veins drain into a descending vein
  which passes through esophageal hiatus anterior
  to esophagus
• Descending vein may connect directly to DV,
  hepatic veins, or IVC
• Mixed (2)- two or more locations

90
What is the Clinical Presentation of an
Unobstructed TAPVC?
91

• Symptoms Tachypnea, mild cyanosis, FTT, feeding
  difficulties, CHF
• PE Increased RV impulse, loud SI, widely split
  and fixed S2 with increased P2, sometimes an S3
  and S4, SEM at ULSB and MDM at LLSB due to
  increased flow across the pulmonary and tricuspid
  valves, respectively
• ECG RAD, RAE, RVH
• CXR Cardiomegaly, increased pulmonary vascular
  markings
• Echocardiography/Doppler/color Doppler Usually
  successful in making diagnosis and confirming
  site of connection
• Cardiac catheterization/cineangiography
• Shows highest saturation at site of pulmonary
  venous connection to the systemic venous system.
• Mild-to-moderate pulmonary hypertension usually
  present. Angiography either in CPV or PA usually
  adequate to confirm diagnosis

92
What is the Clinical Presentation of an
Obstructed TAPVC?
93

• Symptoms occur soon after birth with severe
  cyanosis and respiratory distress and if not
  diagnosed and operated early is highly fatal
• PE severe cyanosis, tachypna, increased RV
  impulse, loud P2, sometimes an S3 and S4. Usually
  no murmur.
• ECG RVH with qR pattern
• CXR Normal to slightly enlarged heart, lung
  fields show reticular pattern of pulmonary venous
  congestion, hyperinflation
• Echocardiography/Doppler/color Doppler Usually
  successful in making diagnosis
• Cardiac catheterization see unobstructed TAPVC

94

• Medical
• Balloon atrial septostomy
• Anticongestive medications
• High calorie formula
• Surgical
• Anastomose CPV to LA
• Close ASD
• Eliminate abnormal connection

95
What is the Definition of and what are the Major
features of a Truncus Arteriosus?
96

• Definition A single arterial trunk leaving the
  heart and giving rise to the aorta, pulmonary
  arteries, and coronary arteries.
• This single arterial trunk over-rides a large
  outlet VSD.

97
What is the Anatomy of a Truncus Arteriousus?
98

• Type I A short MPA gives rise to both branch PAs
• Type II Branch PAs arise adjacent to each other
  from posterior TA
• Type Ill Branch PAs arise from either side of TA
  and are somewhat remote from each other

99
What are the Physical Malformations in TA?
100

• Truncal valve frequently abnormal
• Leaflets thickened and nodular
• May be stenotic, insufficient, or both
• May be bicuspid, tricuspid (most common),
  quadricuspid, or rarely have five or more
  leaflets
• Coronary arteries often abnormal (high origin of
  LCA, single CA)
• Usually no branch PA stenosis

101
What is the Physiology of TA?
102

• Large left-to-right shunt at level of great
  arteries
• Pulmonary and systemic blood flow dependent on
  relative
• Resistances in pulmonary and systemic vascular
  beds

103
What are the Clinical Features of TA?
104

• Symptoms CHF with tachypnea, poor feeding, FTT
• PE Minimal (if any) cyanosis, increased RV
  impulse, ejection click, loud and single S2,
  2-3/6 SEM _at_ LSB, sometimes a diastolic murmur of
  truncal valve insufficiency, bounding pulses, and
  wide pulse pressure (like PDA)
• ECG BVH
• CXR Cardiomegaly, increased pulmonary vascular
  markings, right aortic arch (33)
• Echocardiography/Doppler/color Doppler Easily
  diagnose type of TA, status of truncal valve,
  coronary artery anatomy, and associated lesions
  (i.e. interrupted aortic arch in 20)
• Cardiac catheterization/cineangiography often not
  necessary but may be used to further define
  coronary artery anatomy, BcI exclude additional
  VSD, evaluate distal PAs and aorta, and assess
  pulmonary vascular resistance

105
What is the Management of TA?
106

• Management is surgical
• Removal of PAs from TA and repairing opening in
  PA
• Closure of VSD such the LV supplies the systemic
  arterial circulation and the truncal valve
  becomes the aortic valve
• Establishment of RV-to-PA continuity with a
  cryopreserved valved homograft

107

• Antenataly diagnosed Pulmonary atresia
• Delivered at Vizag at 10 AM
• Went to Chennai for surgery
• Evening surgery was done
• Baby was doing well

108
Pink Baby (L ? R shunt)

• L ? R shunts cause CHF and pulmonary
  hypertension.
• This leads to RV enlargement, RV failure, and cor
  pulmonale.
• These babies present with CHF and respiratory
  distress.
• They are not typically cyanotic.

109
Pink Baby (L ? R shunt)

• These lesions include (among others) ASDs,
  VSDs, and persistently patent ductus arteriosus.

VSD
ASD
110
Pink Baby (L ? R shunt)
Persistently patent ductus arteriosus
111
Pink Baby (L ? R shunt)

• Diagnosing L ? R shunts depends on
• 1. Examination findings
• Non-cyanotic infant in resp distress.
• Crackles, widely-fixed second heart sound,
  elevated JVP, cor pulmonale.
• 2. CXR
• Increased pulmonary vasculature (suggestive of
  CHF).
• RA and/or RV enlargement.
• 3. EKG
• RAE and/or RVH.

112
Pink Baby (L ? R shunt)

• Initial management should be directed at reducing
  the pulm edema.
• Adminster Lasix 1mg/kg IV.
• Peds Cardiology/ PICU should be consulted
  urgently regarding use of
• Morphine
• Nitrates
• Digoxin
• Inotropes

113
Blue Baby (R ? L shunt)

• R ? L shunts cause hypoxia and central cyanosis.
• Neither hypoxia or cyanosis tend to improve with
  100 oxygen.
• R ? L lesions include (among others)
• Tetralogy of Fallot (TOF)
• Transposition of the Great Arteries (TGA)

114
Conclusion

• Routine Neonatal Cardiac surgery is now reality
• Ductus dependent lesion also can be referred
  easily